The Cystic Fibrosis Care, Teaching, and Research Center at Baylor College of Medicine was one of the first such Centers initiated and funded by the NCFRF. This program has been in operation more than a decade with a cystic fibrosis (CF) patient load exceeding 275 patients. The Center is engaged in a clinical, professional, and para-medical teaching program directed to CF and related to pediatric pulmonary problems. Research on the bacteriological flora of the tracheobronchial tract of the CF patients indicated emergence of mucoid Pseudomonas aeruginosa as a significant pathogen for the first time. More recently we have investigated the ciliostatic factor found in the body fluids of CF patients (homozygotes) and CF carriers (heterozygotes) but which is not found in body fluids of normal individuals. The research program outlined in this proposal brings together biochemists and clinicians to identify and characterize the ciliostatic factor (CSF) and to establish its role in the abnormal physiology of cystic fibrosis. Its ultimate objectives are basically twofold: (A) To take advantage of the ciliostatic factor (CSF) to develope a simple, reliable, and reproducible laboratory technique for the detection of the CF carrier, and (B) to determine the true nature of the factor as to whether it is purely a reflection of the disease process or a causative agent. The project is divided into five inter-related parts: (1) Isolation and chemical characterization of the CSF, (2) the effect of the CSF on fibroblast polysaccharide metabolism, (3) the effect of the ciliostatic factor on hormone receptor site assembly in membranes, (4) the role of amine binding to carbohydrases in the action of the factor, and (5) a clinical trial on the usefulness of heparin as an anticiliostatic agent in the reversal of symptoms and complications associated with cystic fibrosis.